FROM PRENATAL DIAGNOSIS TO POSTNATAL MANAGEMENT OF PULMONARY MALFORMATIONS

Key words: Congenital pulmonary airway malformations (CPAM), Prenatal diagnosis, Lung surgery, Infants

Congenital pulmonary airway malformations (CPAM) include a wide spectrum of developmental anomalies. Almost 50% of the cases are diagnosed in utero. These appear as hyperechoic, cystic or mixed lesions. Prenatal clinical course varies from serious complications in utero to polyhydramnios with premature labor. Hydrops and fetal demise is reported in as many as 25-30% of cases. Postnatal presentation ranges from severe respiratory distress to symptoms related to pulmonary hypoplasia, left to right shunting in sequestration or overinflation and pneumothorax. When no prenatal diagnosis is made, children may remain asymptomatic or may present, later in their lives, with complications of recurrent pneumonias or spontaneous pneumothorax. Surgical resection represents the standard for symptomatic cases at birth and later in children with respiratory symptoms. Even though the management for asymptomatic cases remains controversial, early surgical excision to avoid recurrent infections, pneumothorax and malignancy is recommended. New instrumentation and advanced skills make thoracoscopic lobar lung resection the preferred approach.